UBA5 Antibody

說明書

中文名稱：

UBA5兔多克隆抗體
貨號(hào)：

CSB-PA025415GA01HU
規(guī)格：

￥3,900
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

Uniprot No.：

Q9GZZ9
基因名：

UBA5
別名：

5730525G14Rik antibody; AW240750 antibody; FLJ23251 antibody; FLJ23251UBA5 antibody; MGC105565 antibody; ThiFP1 antibody; UBA5 antibody; UBA5 ubiquitin activating enzyme E1 homolog antibody; UBA5_HUMAN antibody; UBE1DC antibody; UBE1DC1 antibody; Ubiquitin activating enzyme 5 antibody; Ubiquitin activating enzyme E1 domain containing 1 antibody; Ubiquitin activating enzyme E1 domain containing protein 1 antibody; Ubiquitin like modifier activating enzyme 5 antibody; Ubiquitin-activating enzyme 5 antibody; Ubiquitin-activating enzyme E1 domain-containing protein 1 antibody; Ubiquitin-like modifier-activating enzyme 5 antibody; UFM1 activating enzyme antibody; UFM1-activating enzyme antibody
宿主：

Rabbit
反應(yīng)種屬：

Human,Mouse,Rat
免疫原：

Human UBA5
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲(chǔ)存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

E1-like enzyme which specifically catalyzes the first step in ufmylation. Activates UFM1 by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a UFM1-E1 thioester and free AMP. Activates UFM1 via a trans-binding mechanism, in which UFM1 interacts with distinct sites in both subunits of the UBA5 homodimer. Trans-binding also promotes stabilization of the UBA5 homodimer, and enhances ATP-binding. Transfer of UFM1 from UBA5 to the E2-like enzyme UFC1 also takes place using a trans mechanism. Ufmylation is involved in reticulophagy (also called ER-phagy) induced in response to endoplasmic reticulum stress. Ufmylation is essential for erythroid differentiation of both megakaryocytes and erythrocytes.
基因功能參考文獻(xiàn)：
1. UFM1 His 70 resembles UBA5 His336 and enters a negatively charged pocked on the other UFM1 molecule. PMID: 28360427
2. These findings explicitly elucidate the role of UBA5 dimerization in UFM1 activation. PMID: 27653677
3. Heterozygous mutations in the UBA5 gene in two sisters with early-onset epileptic encephalopathy . PMID: 28965491
4. Biallelic Variants in UBA5 Link Dysfunctional UFM1 Ubiquitin-like Modifier Pathway to Severe Infantile-Onset Encephalopathy PMID: 27545674
5. clinical, biochemical, and experimental findings support our finding of UBA5 mutations as a pathophysiological cause for early-onset encephalopathies due to abnormal protein ufmylation PMID: 27545681
6. study provides important structural and functional insights into the interaction between UBA5 and Ub-like modifiers, improving the understanding of the biology of the ufmylation pathway. PMID: 26929408
7. The study of UBA5-R246X revealed a dramatically decreased half-life and loss of UFM1 activation due to the absence of the catalytic cysteine Cys250. PMID: 26872069
8. Uba5 residues 364-404 were demonstrated to be necessary for the transthiolation of Ufm1 to Ufc1, and Uba5 381-404 was identified to be the minimal region for Ufc1 recognition PMID: 25084390
9. Crystallization of Uba5 residues 57-363 was performed at 277 K using PEG 3350 as the precipitant, and crystals optimized by microseeding diffracted to 2.95 A resolution, with unit-cell parameters a=b=97.66, c=144.83 A, alpha=beta=90, gamma=120 degrees . PMID: 24915089
10. binding of ATP to Uba5 approximately Ufm1 thioester was required for efficient transfer of Ufm1 from Uba5 to Ufc1 via transthiolation. PMID: 24966333
11. Studies reveal structural features of UBA5 that further understanding of the enzyme reaction mechanism and provide insight into the evolution of ubiquitin activation. PMID: 20368332
12. UBE1DC1 greatly activated SUMO2 in the nucleus or transferred activated-SUMO2 to nucleus after it conjugated SUMO2 in the cytoplasm. PMID: 18442052
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相關(guān)疾病：

Epileptic encephalopathy, early infantile, 44 (EIEE44); Spinocerebellar ataxia, autosomal recessive, 24 (SCAR24)
亞細(xì)胞定位：

Cytoplasm. Nucleus. Endoplasmic reticulum membrane. Golgi apparatus.
蛋白家族：

Ubiquitin-activating E1 family, UBA5 subfamily
組織特異性：

Widely expressed.
數(shù)據(jù)庫鏈接：

HGNC: 23230

OMIM: 610552

KEGG: hsa:79876

STRING: 9606.ENSP00000348565

UniGene: Hs.170737

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